Intermediate uveitis: pattern of etiology, complications, treatment and outcome in a tertiary academic center

Abstract

BackgroundPatients with intermediate uveitis (IU) represent a heterogenous group characterized by a wide spectrum of etiologies and regional differences. Aim of the study was to analyze the characteristics of patients with IU examined in an academic center in Germany.MethodsWe conducted a retrospective analysis of the clinical records of all patients with intermediate uveitis referred to the Eye Center, University of Freiburg from 2007 to 2014. Diagnosis followed the Standardization in Uveitis Nomenclature (SUN) criteria. Data analysis included: etiology of IU, demographics, complications, treatment and visual acuity.ResultsWe identified 159 patients with intermediate uveitis during that period. Mean age at diagnosis was 35 years. Most are female (64%), and the mean duration of IU was 6.1 years (range 1 month – 35 years). Etiology of IU was idiopathic in 59%. Multiple sclerosis (MS) (20%) and sarcoidosis (10%) were frequent systemic causes of IU. Other etiologies including infectious diseases (tuberculosis, borreliosis) or immune-mediated conditions (eg, after vaccination) were present in 11%. The pattern of complications included macular edema (CME) (36%), cataract (24%), secondary glaucoma (7%), and epiretinal membrane formation (19%). Periphlebitis and optic neuritis were more frequent in conjunction with MS. Treatment comprised local and systemic steroids, immunosuppressive agents, biologics, and surgery. Best corrected visual acuity was better than 20/25 in 60% of the eyes after more than 10 years of follow-up.ConclusionsIn our German academic center, most IU cases were idiopathic or associated with MS or sarcoidosis. In contrast to other countries, infectious cases were rare. Patients’ overall visual prognosis is favorable even when the duration of IU has been long and and despite numerous complications.

Ocular Sarcoidosis

“…53 Intermediate uveitis usually affects young patients and is idiopathic. 25,57 This pattern of uveitis is seen in only a minority of patients with sarcoid uveitis (6-19%). 11, 34,36,58 On the other hand, sarcoidosis is responsible for 7 to 18% of the cases of intermediate uveitis, one of the two most important systemic associations along with multiple sclerosis.…”

“…11, 34,36,58 On the other hand, sarcoidosis is responsible for 7 to 18% of the cases of intermediate uveitis, one of the two most important systemic associations along with multiple sclerosis. 25,57 Vitreous opacities may comprise "snow-balls" and/or "string of pearls" (►Fig. 3) and these are highly suggestive of a granulomatous process.…”

Uveitis intermedia

“…Sie verläuft in den allermeisten Fällen (> 90 %) chronisch [3]. Am häufigsten sind junge Erwachsene betroffen, wobei das Durchschnittsalter bei Erstdiagnose zwischen 23 und 33 Jahren liegt [1].…”

“…Der ältere Begriff der Pars planitis bezeichnet eine Unterform der intermediären Uveitis, bei der sogenannte "Snowballs" und "Snowbanks" auftreten und keine systemische Grunderkrankung oder infektiöse Ursache gefunden wird [6,7]. In 37-60 % der intermediären Uveitiden kommt es zur Entwicklung eines zystoiden Makulaödems, welches gleichzeitig die häufigste Ursache für eine Sehverschlechterung ist [1,8]. Genauso wie die Papillitis gilt das Makulaödem als therapiebedürftige Komplikation.…”

Disease Remission in Children and Adolescents with Intermediate Uveitis: A Survival Analysis

“…Intermediate uveitis (IU) is characterized by inflammation of the anterior vitreous, peripheral retina, and ciliary body [1, 2]. Although in most cases it is an idiopathic condition, it can be associated with several diseases, such as multiple sclerosis, sarcoidosis, or Lyme’s disease [3-7].…”